Abnormal fatty acid metabolism in spinal muscular atrophy may predispose to perioperative risks.
Journal Article (Journal Article)
A 15 year old boy with SMA type II underwent spinal fusion and suffered a mitochondrial Reye-like catabolic crisis 4 days postop with hypoketotic hypoglycemia, lactic acidaemia, hyperammonemia and liver failure, with 90% coagulative necrosis and diffuse macro- and microvesicular steatosis, requiring orthotopic liver transplantation. This crisis responded in part to mitochondrial therapy and anabolic rescue. He made a dramatic sustained neurological recovery, though his post-transplant liver biopsies revealed micro- and macrosteatosis. We hypothesize that a combination of surgical stress-catecholamine induced lipolysis, prolonged general anaesthesia with propofol and sevoflurane, and perioperative fasting on a background of decreased β-oxidation were potential risk factors for the mitochondrial decompensation.
Full Text
Duke Authors
Cited Authors
- Zolkipli, Z; Sherlock, M; Biggar, WD; Taylor, G; Hutchison, JS; Peliowski, A; Alman, BA; Ling, SC; Tein, I
Published Date
- September 2012
Published In
Volume / Issue
- 16 / 5
Start / End Page
- 549 - 553
PubMed ID
- 22264649
Electronic International Standard Serial Number (EISSN)
- 1532-2130
Digital Object Identifier (DOI)
- 10.1016/j.ejpn.2012.01.004
Language
- eng
Conference Location
- England